Saturday, October 13, 2012

Orbital Rhabdomyosarcoma

9 years boy
Proptosis left eye - 3 months
Drooping of left upper eye lid - 2 months
On Examination
         Left ptosis with proptosis
         Fundii normal
         EOM - restricted in superior & lateral direction [left eye]
         Va – Normal
         Vf – Normal in right eye
Pre Op CT Scan

Pre Op MRI

Pre Op MRI

Left frontal trephine craniotomy, extradural superior orbital approach and microsurgical excision of tumor.
Extraconal well defined, non-encapsulated, soft, mild vascular, whitish grey tumor attached to levator muscle.
Complete excision done
Post Op
Left ptosis
Decreased proptosis
Left superior rectus paresis
Subconjuctival hemorrhage

  • Rhabdomyosarcoma - Most common pediatric soft tissue sarcomas  (approximately 50%)
  • Peak age 2- 5 years
  • Male preponderance (1.4:1)
  • 1/3rd  of RMS patients have other congenital abnormalities
    • GI, GU, CV, CNS
  • Majority cases sporadic; but some associated with genetic conditions
    • Li Fraumeni (p53 mutation)
    • NF 1
    • Beckwith - Wiedemann
Pattern of spread
  • Locally invasive tumor often with pseudocapsule
  • Potential for local spread along fascial or muscle planes, lymphatic extension and hematogenous dissemination
  • Regional lymphatic & hematogenous metastases -15%
Prognostic Factors
  • Histology
  • Stage
    • Primary site (most important)
    • Tumor Size
    • LN involvement
    • Metastatic disease
  • Group
    • Extent of resection
  • Age
    • < 1
    • >10
    • Skull base erosion, CN palsy & Intracranial extension
  • Gross disease
    • Soft, fleshy tumors with variation in extent of invasion and necrosis
  • IHC stains to ascertain muscle of origin
    • Antidesmin, antivimentin,  anti-muscle specific actin
    • Anti-Myo D
  • Embryonal
    • Most common
    • 60-70% of all childhood RMS
  • Boytroid
  • Spindle cell
  • Alveolar
  • Undifferentiated/pleomorphic
Histology and Survival

Clinical presentation
  • Asymptomatic mass
  • Rapid growing painless mass
  • Mass effect on associated organs and tissues
  • Commonest site orbit > parameningeal sites > other sites
Staging System
Intergroup Rhabdomyosarcoma Study Clinical Grouping Classification

Prognostic Factors
§  favorable prognostic site
§  lymphatic extension/hematogenous metastasis is rare
§  embryonal histology

    • Surgical procedure either excision/biopsy
    • Chemo-radiation
    • Cure rates of  > 90%

Wednesday, April 25, 2012

Left Petroclival Lesion

1.    Double vision-1 year
2.    Tingling over face-4 months

1.    Left corneal reflex absent
2.    Left 6th nerve palsy


Left temporal trephine craniotomy with subtemporal intradural approach with tumor excision done

No fresh episodes, left 5th and 6th nerve impairment persisted

NCCT Head:-

5TH Nerve shwannoma